Specifications

Usage & Assay Information

Storage & Safety

Storage

Store at 4 deg C for short term storage. Aliquot and store at -20 deg C for long term storage. Avoid repeated freeze/thaw cycles.

General Information

Biological Significance

The prion protein is a large membrane protein that occurs normally in neurons of the human brain and is thought to be involved in synaptic transmission. In prion diseases, such as Creutzfeld-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome (GSS), Fatal Familial Insomnia (FFI), Alpers Syndrome and Kuru, the normal cellular form of this protein (PrPc) is transformed into an altered protein when it comes into contact with an infectious prion protein (PrPsc) from another host. This altered PrPsc accumulates in cytoplasmic vesicles of diseased individuals forming lesions, vacuoles and amyloid deposits.